scholarly journals Syringobulbia in pediatric patients with Chiari malformation type I

2018 ◽  
Vol 22 (1) ◽  
pp. 52-60 ◽  
Author(s):  
Arnold H. Menezes ◽  
Jeremy D. W. Greenlee ◽  
Brian J. Dlouhy
Keyword(s):  
Posterior Fossa ◽  
Cranial Nerve ◽  
Chiari Malformation ◽  
Fourth Ventricle ◽  
Pediatric Patients ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Presenting Symptoms ◽  
Cranial Nerve Palsies

OBJECTIVESyringobulbia (SB) is a rare entity, with few cases associated with Chiari malformation type I (CM-I) in the pediatric population. The authors reviewed all pediatric cases of CM-I–associated SB managed at their institution in order to better understand the presentation, treatment, and surgical outcomes of this condition.METHODSA prospectively maintained institutional database of craniovertebral junction abnormalities was analyzed to identify all cases of CM-I and SB from the MRI era (i.e., after 1984). The authors recorded presenting symptoms, physical examination findings, radiological findings, surgical treatment strategy, intraoperative findings, and outcomes. SB cases associated with tumors, infections, or type II Chiari malformations were excluded.RESULTSThe authors identified 326 pediatric patients with CM-I who were surgically treated. SB was identified in 13 (4%) of these 326 patients. Headache and neck pain were noted in all 13 cases. Cranial nerve abnormalities were common: vagus and glossopharyngeal nerve dysfunction was the most frequent observation. Other cranial nerves affected included the trigeminal, abducens, and hypoglossal nerves. Several patients exhibited multiple cranial nerve palsies at presentation. Central sleep apnea was present in 6 patients.Syringomyelia (SM) was present in all 13 patients. SB involved the medulla in all cases, and extended rostrally into the pons and midbrain in 2 patients; in 1 of these 2 cases the cavity extended further rostrally to the cerebrum (syringocephaly). SB communicated with the fourth ventricle in 7 of the 13 cases.All 13 patients were treated with posterior fossa decompression with intradural exploration to ensure CSF egress out of the fourth ventricle and through the foramen magnum. The foramen of Magendie was found to be occluded by an arachnoid veil in 9 cases. Follow-up evaluation revealed that SB improved before SM. Cranial nerve palsies regressed in 11 of the 13 patients, and SB improved in all 13.CONCLUSIONSThe incidence of SB in our surgical series of pediatric patients with CM-I was 4%, and all of these patients had accompanying SM. The SB cavity involved the medulla in all cases and was found to communicate with the fourth ventricle in 54% of cases. Posterior fossa decompression with intradural exploration and duraplasty is an effective treatment for these patients.

Syringobulbia in a Pediatric Population

Neurosurgery ◽  
2005 ◽  
Vol 57 (6) ◽  
pp. 1147-1153 ◽  
Author(s):  
Jeremy D.W. Greenlee ◽  
Arnold H. Menezes ◽  
Bryan A. Bertoglio ◽  
Kathleen A. Donovan
Keyword(s):  
Posterior Fossa ◽  
Cranial Nerve ◽  
Fourth Ventricle ◽  
Pediatric Population ◽  
Demographic Data ◽  
Posterior Fossa Decompression ◽  
Radiographic Findings ◽  
Presenting Symptoms ◽  
Cranial Nerve Dysfunction ◽  
Cranial Nerve Palsies

Abstract OBJECTIVE: To better understand the presentation, management, and outcome of syringobulbia in the pediatric age group. METHODS: The University of Iowa pediatric neurosurgery database was searched for patients under the age of 18 with a diagnosis of syringobulbia. The patients' records were retrospectively reviewed for demographic data, chief complaint and presenting symptoms, neurological and radiographic findings, treatment, outcome, and complications. Children with open neural tube defects and Chiari II malformations were excluded. RESULTS: Six pediatric patients were identified as meeting inclusion criteria. The average age at time of surgery was 14.8 years. The chief complaints were vision impairment in three children and numbness, gait instability, and headache worsened with Valsalva in one patient each. Other prominent symptoms included sleep apnea and weakness. All patients showed at least one cranial nerve dysfunction. Radiographs revealed hindbrain herniation and associated syringomyelia in all cases. Two patients had scoliosis. Treatment was posterior fossa decompression with cerebellar tonsillar shrinkage, opening of foramen of Magendie, and duraplasty. Two patients also required concomitant ventral decompression. The cavity of syringobulbia communicated with syringomyelia and the fourth ventricle in most children but was distinct from the fourth ventricle. Two patients received fourth ventricle to subarachnoid shunts. Follow-up averaged 3.2 years, and all patients clinically improved after surgery. Magnetic resonance imaging documented resolution of syringobulbia in all cases, with syringomyelia improving in all cases. There was no permanent morbidity or mortality in the series. CONCLUSION: Syringobulbia is strongly associated with Chiari malformation and syringomyelia, and patients often present because of cranial nerve palsies. Posterior fossa decompression is a safe and effective treatment.

scholarly journals Management of hydrocephalus and subdural hygromas in pediatric patients after decompression of Chiari malformation type I: case series and review of the literature

2018 ◽  
Vol 22 (4) ◽  
pp. 426-438 ◽  
Author(s):  
Andrew C. Vivas ◽  
Nir Shimony ◽  
Eric M. Jackson ◽  
Risheng Xu ◽  
George I. Jallo ◽  
...  
Keyword(s):  
Literature Review ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Pediatric Patients ◽  
High Dose ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Dural Graft ◽  
Csf Diversion

OBJECTIVEHydrocephalus associated with subdural hygromas is a rare complication after decompression of Chiari malformation type I (CM-I). There is no consensus for management of this complication. The authors present a series of 5 pediatric patients who underwent CM-I decompression with placement of a dural graft complicated by posterior fossa hygromas and hydrocephalus that were successfully managed nonoperatively.METHODSA retrospective review over the last 5 years of patients who presented with hydrocephalus and subdural hygromas following foramen magnum decompression with placement of a dural graft for CM-I was conducted at 2 pediatric institutions. Their preoperative presentation, perioperative hospital course, and postoperative re-presentation are discussed with attention to their treatment regimen and ultimate outcome. In addition to reporting these cases, the authors discuss all similar cases found in their literature review.RESULTSOver the last 5 years, the authors have encountered 194 pediatric cases of CM-I decompression with duraplasty equally distributed at the 2 institutions. Of those cases, 5 pediatric patients with a delayed postoperative complication involving hydrocephalus and subdural hygromas were identified. The 5 patients were managed nonoperatively with acetazolamide and high-dose dexamethasone; dosages of both drugs were adjusted to the age and weight of each patient. All patients were symptom free at follow-up and exhibited resolution of their pathology on imaging. Thirteen similar pediatric cases and 17 adult cases were identified in the literature review. Most reported cases were treated with CSF diversion or reoperation. There were a total of 4 cases previously reported with successful nonoperative management. Of these cases, only 1 case was reported in the pediatric population.CONCLUSIONSDe novo hydrocephalus, in association with subdural hygromas following CM-I decompression, is rare. This presentation suggests that these complications after posterior fossa decompression with duraplasty can be treated with nonoperative medical management, therefore obviating the need for CSF diversion or reoperation.

scholarly journals Expansile duraplasty and obex exploration compared with bone-only decompression for Chiari malformation type I in children: retrospective review of outcomes and complications

2021 ◽  
Vol 27 (1) ◽  
pp. 1-8
Author(s):  
Chibawanye I. Ene ◽  
Anthony C. Wang ◽  
Kelly L. Collins ◽  
Robert H. Bonow ◽  
Lynn B. McGrath ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Posterior Fossa ◽  
Retrospective Review ◽  
Chiari Malformation ◽  
Pediatric Patients ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Symptom Resolution ◽  
Presenting Symptoms ◽  
Significant Difference

OBJECTIVEWhile a select population of pediatric patients with Chiari malformation type I (CM-I) remain asymptomatic, some patients present with tussive headaches, neurological deficits, progressive scoliosis, and other debilitating symptoms that necessitate surgical intervention. Surgery entails a variety of strategies to restore normal CSF flow, including increasing the posterior fossa volume via bone decompression only, or bone decompression with duraplasty, with or without obex exploration. The indications for duraplasty and obex exploration following bone decompression remain controversial. The objective of this study was to describe an institutional series of pediatric patients undergoing surgery for CM-I, performed by a single neurosurgeon. For patients presenting with a syrinx, the authors compared outcomes following bone-only decompression with duraplasty only and with duraplasty including obex exploration. Clinical outcomes evaluated included resolution of syrinx, scoliosis, presenting symptoms, and surgical complications.METHODSA retrospective review was conducted of the medical records of 276 consecutive pediatric patients with CM-I operated on at a single institution between 2001 and 2015 by the senior author. Imaging findings of tonsillar descent, associated syrinx (syringomyelia or syringobulbia), basilar invagination, and clinical assessment of CM-I–attributable symptoms and scoliosis were recorded. In patients presenting with a syrinx, clinical outcomes, including syrinx resolution, symptom resolution, and impact on scoliosis progression, were compared for three surgical groups: bone-only/posterior fossa decompression (PFD), PFD with duraplasty (PFDwD), and PFD with duraplasty and obex exploration (PFDwDO).RESULTSPFD was performed in 25% of patients (69/276), PFDwD in 18% of patients (50/276), and PFDwDO in 57% of patients (157/276). The mean follow-up was 35 ± 35 months. Nearly half of the patients (132/276, 48%) had a syrinx. In patients presenting with a syrinx, PFDwDO was associated with a significantly higher likelihood of syrinx resolution relative to PFD only (HR 2.65, p = 0.028) and a significant difference in time to symptom resolution (HR 2.68, p = 0.033). Scoliosis outcomes did not differ among treatment groups (p = 0.275). Complications were not significantly higher when any duraplasty (PFDwD or PFDwDO) was performed following bone decompression (p > 0.99).CONCLUSIONSIn this series of pediatric patients with CM-I, patients presenting with a syrinx who underwent expansile duraplasty with obex exploration had a significantly greater likelihood of syrinx and symptom resolution, without increased risk of CSF-related complications, compared to those who underwent bone-only decompression.

scholarly journals Syrinx resolution after posterior fossa decompression in patients with scoliosis secondary to Chiari malformation type I

2011 ◽  
Vol 21 (6) ◽  
pp. 1143-1150 ◽  
Author(s):  
Tao Wu ◽  
Zezhang Zhu ◽  
Jian Jiang ◽  
Xin Zheng ◽  
Xu Sun ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I

Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation Type I in pediatric patients: a meta-analysis

2008 ◽  
Vol 2 (1) ◽  
pp. 42-49 ◽  
Author(s):  
Susan R. Durham ◽  
Kristina Fjeld-Olenec
Keyword(s):  
Cerebrospinal Fluid ◽  
Surgical Treatment ◽  
Clinical Improvement ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Meta Analysis ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Patient Age

Object Surgery for Chiari malformation Type I (CM-I) is one of the most common neurosurgical procedures performed in children, although there is clearly no consensus among practitioners about which surgical method is preferred. The objective of this meta-analysis was to compare the outcome of posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression without duraplasty (PFD) for the treatment of CM-I in children. Methods The authors searched Medline–Ovid, The Cochrane Library, and the conference proceedings of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons (2000–2007) for studies meeting the following inclusion criteria: 1) surgical treatment of CM-I; 2) surgical techniques of PFD and PFDD being reported in a single cohort; and 3) patient age < 18 years. Results Five retrospective and 2 prospective cohort studies involving a total of 582 patients met the criteria for inclusion in the meta-analysis. Of the 582 patients, 316 were treated with PFDD and 266 were treated with PFD alone. Patient age ranged from 6 months to 18 years. Patients undergoing PFDD had a significantly lower reoperation rate (2.1 vs 12.6%, risk ratio [RR] 0.23, 95% confidence interval [CI] 0.08–0.69) and a higher rate of cerebrospinal fluid–related complications (18.5 vs 1.8%, RR 7.64, 95% CI 2.53–23.09) than those undergoing PFD. No significant differences in either clinical improvement (78.6 vs 64.6%, RR 1.23, 95% CI 0.95–1.59) or syringomyelia decrease (87.0 vs 56.3%, RR 1.43, 95% CI 0.91–2.25) were noted between PFDD and PFD. Conclusions Posterior fossa decompression with duraplasty is associated with a lower risk of reoperation than PFD but a greater risk for cerebrospinal fluid–related complications. There was no significant difference between the 2 operative techniques with respect to clinical improvement or decrease in syringomyelia.

Patients with Chiari malformation Type I presenting with acute neurological deficits: case series

2011 ◽  
Vol 7 (3) ◽  
pp. 244-247 ◽  
Author(s):  
Chester K. Yarbrough ◽  
Alexander K. Powers ◽  
Tae Sung Park ◽  
Jeffrey R. Leonard ◽  
David D. Limbrick ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Posterior Fossa ◽  
Neurological Deficit ◽  
Chiari Malformation ◽  
Acute Onset ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Sensory Deficits ◽  
Operative Notes

Object A subset of patients with Chiari malformation Type I (CM-I) presented with acute onset of a neurological deficit. In this study the authors summarize their experience with these patients' clinical presentation, imaging results, timing of surgery, and outcome following decompression. Methods The authors reviewed clinical records, imaging studies, and operative notes from all patients undergoing posterior fossa decompression for CM-I at St. Louis Children's Hospital from 1990 to 2008. Of the 189 patients who underwent surgery, 6 were identified with the acute onset of a neurological deficit at presentation. Results All 6 children (age range 3–14 years, 3 boys and 3 girls) had either syringomyelia (5 patients) or T2 signal changes in the spinal cord (1 patient) and CM-I on initial MR imaging. Three patients presented after minor trauma (1 with paraparesis, 2 with sensory deficits). Three patients presented without a clear history of trauma (1 with abrupt onset of spontaneous dysphagia and ataxia, 2 with sensory deficits). Decompression was performed at a mean 7.7 ± 4.9 days after symptom onset (7.0 ± 1.6 days after neurosurgical evaluation). In 1 patient, symptoms had resolved by the time of surgery; in the remainder of the patients, clear improvements were noted within 2 weeks of surgery, with complete resolution of symptoms by 12 months postoperatively. Follow-up MR images were obtained in 4 patients, demonstrating improvement in the extent of the syrinx in each patient. Conclusions Children with CM-I and syringomyelia can develop acute spinal cord or bulbar deficits with relatively minor head or neck injuries. The prognosis for symptomatic improvement in the observed deficit is good, with each patient in our series showing resolution of deficits over time. However, based on this relatively limited experience, the authors suggest that patients who present with an acute neurological deficit and are found to have CM-I be managed with early posterior fossa decompression. Patients with CM-I and syringomyelia may be at higher risk of acute neurological deficit than those without a syrinx.

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